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The Neurodevelopmental Outcomes of Patients With Single Ventricles Across the Lifespan

Karin du Plessis1, Yves d'Udekem2

  • 1Murdoch Children's Research Institute, Melbourne, Victoria, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia.

The Annals of Thoracic Surgery
|July 10, 2019
PubMed

Insights

Neurodevelopmental impairments affect many single-ventricle patients, with innate factors playing a key role. Further research is needed to understand lifelong impacts and improve interventions for these patients.

Area of Science:

  • Pediatric Cardiology
  • Neurodevelopmental Disorders
  • Congenital Heart Disease

Background:

  • One-third of single-ventricle patients experience moderate to severe neurodevelopmental impairments.
  • These impairments represent a significant comorbidity impacting quality of life.

Purpose of the Study:

  • To review neurodevelopment across the lifespan in single-ventricle patients.
  • To identify contributing factors and current interventions.
  • To highlight gaps in knowledge and future research directions.

Main Methods:

  • A focused literature review was conducted.
  • Articles in English from 1980 to 2018 were analyzed.

Main Results:

  • Innate patient factors, not surgical factors, influence neurodevelopmental outcomes.
  • Current outcome measures have limitations, and adult/longitudinal data are scarce.
  • Adults may face abnormal brain aging and early-onset dementia; intervention efficacy is understudied.

Conclusions:

  • Improved neurodevelopmental testing is needed for accurate future functioning prediction.
  • Multidisciplinary care is essential for implementing effective interventions.
  • Psychological and educational interventions may benefit patients with Fontan circulation.
Abstract

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